Adrenocortical carcinoma

Tumor - adrenal

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Adrenocortical carcinoma is a cancer of the adrenal glands.

Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).

This CT scan of the upper abdomen shows an adrenal metastasis (spreading of a tumor to the adrenal gland, above the kidney) in a person with lung cancer.

CT scan of the upper abdomen in a person with a right adrenal mass. The adrenal glands are located above the kidneys.

Causes

Adrenocortical carcinoma is most common in children younger than 5 years old and adults in their 30s and 40s.

The condition may be linked to a cancer syndrome that is passed down through families (inherited). Both men and women can develop this tumor.

Adrenocortical carcinoma can produce the hormones cortisol, aldosterone, estrogen, or testosterone, as well as other hormones. In women the tumor often releases these hormones, which can lead to male characteristics.

Adrenocortical carcinoma is very rare. The cause is unknown. About 2 people per million develop this type of tumor.

Symptoms

Symptoms of increased cortisol or other adrenal gland hormones:

Fatty, rounded hump high on the back just below the neck (buffalo hump)

Virilization - the appearance of male characteristics, including increased body hair (especially on the face), pubic hair, acne, deepening of the voice, and enlarged clitoris (girls)

Symptoms of increased aldosterone are the same as symptoms of low potassium, and include:

Muscle cramps

Weakness

Exams and Tests

The doctor or nurse will examine you. You may have high blood pressure and changes in body shape, such as breast growth in men (gynecomastia) or male signs in women (virilization). Blood tests will be done to check hormone levels:

Treatment

Primary treatment is surgery to remove the tumor. Adrenocortical carcinoma may not improve with chemotherapy. Medications may be given to reduce production of cortisol, which causes many of the symptoms.

Outlook (Prognosis)

The outcome depends on how early the diagnosis is made and whether the tumor has spread (metastasized). Tumors that have spread usually lead to death within 1 to 3 years.

Possible Complications

The tumor can spread to the liver, bone, lung, or other areas.

When to Contact a Medical Professional

Call your health care provider if you or your child has symptoms of adrenocortical carcinoma, Cushing syndrome, or failure to grow.